Treatment of gastrointestinal disease in systemic sclerosis (scleroderma)
- Stephanie A Kaye-Barrett, MD
Stephanie A Kaye-Barrett, MD
- Consultant and Honorary Lecturer in Rheumatology
- The Chelsea and Westminster Hospital
- Christopher P Denton, MD
Christopher P Denton, MD
- Professor of Experimental Rheumatology
- Royal Free Hospital, London
- Section Editors
- John S Axford, DSc, MD, FRCP, FRCPCH
John S Axford, DSc, MD, FRCP, FRCPCH
- Section Editor — Scleroderma
- Emeritus Professor of Rheumatology
- St George's University of London
- Nicholas J Talley, MD, PhD
Nicholas J Talley, MD, PhD
- Section Editor — Motility Disorders
- Professor of Medicine, University of Newcastle, Australia
- Adjunct Professor of Medicine and Epidemiology and Consultant, Mayo Clinic, Rochester, MN
- Adjunct Professor, University of North Carolina
- Deputy Editors
- Monica Ramirez Curtis, MD, MPH
Monica Ramirez Curtis, MD, MPH
- Deputy Editor — Rheumatology
- Instructor of Medicine
- Harvard Medical School
- Shilpa Grover, MD, MPH, AGAF
Shilpa Grover, MD, MPH, AGAF
- Deputy Editor — Gastroenterology/Hepatology
- Assistant Professor of Medicine, Part-time
- Harvard Medical School
Nearly 90 percent of patients with systemic sclerosis (SSc) have some degree of gastrointestinal (GI) involvement, and approximately one-half are symptomatic [1,2]. Although the esophagus is the most frequently affected part of the GI tract, any part of the GI tract may be involved. Severe involvement occurs in less than 10 percent of patients and is associated with an unfavorable prognosis [3,4]. This topic will review the management of GI disease associated with SSc. Our approach is generally consistent with clinical practice guidelines for the management of common SSc manifestations affecting the GI tract . The pathogenesis, pathology, clinical manifestations, and diagnosis of GI manifestations of SSc are discussed separately. (See "Gastrointestinal manifestations of systemic sclerosis (scleroderma)".)
The management of oral manifestations of systemic sclerosis (SSc) is largely supportive. Regular dental hygiene is necessary to help prevent dental caries. Patients with decreased oral aperture may be able to improve gingival help using adaptive oral hygiene devices (eg, flossers, oscillating-rotating toothbrushes) combined with facial exercises to increase the oral mouth opening [6,7]. Artificial saliva may be used in patients who also have sicca syndrome. (See "Treatment of dry mouth and other non-ocular sicca symptoms in Sjögren's syndrome", section on 'Saliva substitutes'.)
Management of esophageal disease is largely directed toward the amelioration of symptoms of heartburn and dysphagia, which may be due to esophageal dysmotility or gastroesophageal reflux disease (GERD) and its complications (eg, strictures, esophagitis).
Gastroesophageal reflux — The management of GERD in patients with systemic sclerosis (SSc) is similar to that in patients without SSc. However, patients with SSc often have more severe symptoms.
While lifestyle and dietary modification should be recommended in all patients with GERD, these measures are usually inadequate by themselves. These measures include elevation of the head of the bed and selective elimination of dietary triggers (see "Medical management of gastroesophageal reflux disease in adults", section on 'Lifestyle and dietary modification'). Calcium channel blockers and anticholinergic agents, which may be used to treat other manifestations of SSc, can potentially worsen reflux.
- Turner R, Lipshutz W, Miller W, et al. Esophageal dysfunction in collagen disease. Am J Med Sci 1973; 265:191.
- Akesson A, Wollheim FA. Organ manifestations in 100 patients with progressive systemic sclerosis: a comparison between the CREST syndrome and diffuse scleroderma. Br J Rheumatol 1989; 28:281.
- Cohen S. The gastrointestinal manifestations of scleroderma: pathogenesis and management. Gastroenterology 1980; 79:155.
- Steen VD, Medsger TA Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 2000; 43:2437.
- Hansi N, Thoua N, Carulli M, et al. Consensus best practice pathway of the UK scleroderma study group: gastrointestinal manifestations of systemic sclerosis. Clin Exp Rheumatol 2014; 32:S.
- Jung S, Martin T, Schmittbuhl M, Huck O. The spectrum of orofacial manifestations in systemic sclerosis: a challenging management. Oral Dis 2016.
- Balzer J. The use of adaptive oral hygiene devices and orofacial exercise by adults with systemic sclerosis (scleroderma) seems to improve their gingival health. J Evid Based Dent Pract 2012; 12:97.
- Hendel L, Hage E, Hendel J, Stentoft P. Omeprazole in the long-term treatment of severe gastro-oesophageal reflux disease in patients with systemic sclerosis. Aliment Pharmacol Ther 1992; 6:565.
- Hendel L. Hydroxyproline in the oesophageal mucosa of patients with progressive systemic sclerosis during omeprazole-induced healing of reflux oesophagitis. Aliment Pharmacol Ther 1991; 5:471.
- Hendel L, Aggestrup S, Stentoft P. Long-term ranitidine in progressive systemic sclerosis (scleroderma) with gastroesophageal reflux. Scand J Gastroenterol 1986; 21:799.
- Carlson DA, Hinchcliff M, Pandolfino JE. Advances in the evaluation and management of esophageal disease of systemic sclerosis. Curr Rheumatol Rep 2015; 17:475.
- Mansour KA, Malone CE. Surgery for scleroderma of the esophagus: a 12-year experience. Ann Thorac Surg 1988; 46:513.
- Kent MS, Luketich JD, Irshad K, et al. Comparison of surgical approaches to recalcitrant gastroesophageal reflux disease in the patient with scleroderma. Ann Thorac Surg 2007; 84:1710.
- Kahan A, Chaussade S, Gaudric M, et al. The effect of cisapride on gastro-oesophageal dysfunction in systemic sclerosis: a controlled manometric study. Br J Clin Pharmacol 1991; 31:683.
- Horowitz M, Maddern GJ, Maddox A, et al. Effects of cisapride on gastric and esophageal emptying in progressive systemic sclerosis. Gastroenterology 1987; 93:311.
- Johnson DA, Drane WE, Curran J, et al. Metoclopramide response in patients with progressive systemic sclerosis. Effect on esophageal and gastric motility abnormalities. Arch Intern Med 1987; 147:1597.
- Feldman M, Smith HJ. Effect of cisapride on gastric emptying of indigestible solids in patients with gastroparesis diabeticorum. A comparison with metoclopramide and placebo. Gastroenterology 1987; 92:171.
- Ehrenpreis ED, Roginsky G, Alexoff A, Smith DG. Domperidone is Commonly Prescribed With QT-Interacting Drugs: Review of a Community-based Practice and a Postmarketing Adverse Drug Event Reporting Database. J Clin Gastroenterol 2017; 51:56.
- Fiorucci S, Distrutti E, Bassotti G, et al. Effect of erythromycin administration on upper gastrointestinal motility in scleroderma patients. Scand J Gastroenterol 1994; 29:807.
- Fiorucci S, Distrutti E, Gerli R, Morelli A. Effect of erythromycin on gastric and gallbladder emptying and gastrointestinal symptoms in scleroderma patients is maintained medium term. Am J Gastroenterol 1994; 89:550.
- Wysowski DK, Bacsanyi J. Cisapride and fatal arrhythmia. N Engl J Med 1996; 335:290.
- Hendel L, Svejgaard E, Walsøe I, et al. Esophageal candidosis in progressive systemic sclerosis: occurrence, significance, and treatment with fluconazole. Scand J Gastroenterol 1988; 23:1182.
- Schulz SW, O'Brien M, Maqsood M, et al. Improvement of severe systemic sclerosis-associated gastric antral vascular ectasia following immunosuppressive treatment with intravenous cyclophosphamide. J Rheumatol 2009; 36:1653.
- Parodi A, Sessarego M, Greco A, et al. Small intestinal bacterial overgrowth in patients suffering from scleroderma: clinical effectiveness of its eradication. Am J Gastroenterol 2008; 103:1257.
- Rezaie A, Pimentel M, Rao SS. How to Test and Treat Small Intestinal Bacterial Overgrowth: an Evidence-Based Approach. Curr Gastroenterol Rep 2016; 18:8.
- Shah SC, Day LW, Somsouk M, Sewell JL. Meta-analysis: antibiotic therapy for small intestinal bacterial overgrowth. Aliment Pharmacol Ther 2013; 38:925.
- Butt SK, Alam A, Cohen R, et al. Lack of effect of sacral nerve stimulation for incontinence in patients with systemic sclerosis. Colorectal Dis 2015; 17:903.
- Thaha MA, Abukar AA, Thin NN, et al. Sacral nerve stimulation for faecal incontinence and constipation in adults. Cochrane Database Syst Rev 2015; :CD004464.
- Kenefick NJ, Vaizey CJ, Nicholls RJ, et al. Sacral nerve stimulation for faecal incontinence due to systemic sclerosis. Gut 2002; 51:881.
- Glasgow SC, Lowry AC. Long-term outcomes of anal sphincter repair for fecal incontinence: a systematic review. Dis Colon Rectum 2012; 55:482.
- OROPHARYNGEAL DISEASE
- ESOPHAGEAL DISEASE
- Gastroesophageal reflux
- Esophageal motility disorder
- Infectious and medication-induced esophagitis
- GASTRIC DISEASE
- Gastric antral venous ectasia
- SMALL INTESTINAL DISEASE
- Small intestinal bacterial overgrowth
- Dysmotility and intestinal pseudo-obstruction
- Pneumatosis cystoides intestinalis and pneumoperitoneum
- COLONIC AND ANORECTAL DISEASE
- Fecal incontinence
- LIVER AND BILIARY TREE INVOLVEMENT
- PANCREATIC DISEASE
- SUMMARY AND RECOMMENDATIONS